A 25-month-old, Caucasian girl presented for evaluation of high myopia, decreased vision, new onset nystagmus and anomalous head posture (AHP). The parents noted decreased vision, nystagmus, photophobia, nycloptia and AHP 8-10 weeks prior to examination. The patient had an uncomplicated prenatal and birth history with subsequent normal growth and development. She was healthy other than a history of uncomplicated otitis media. There was no history of trauma, recent illness or new medications. Her family history was notable for myopia, amblyopia, and a paternal cousin diagnosed with “optic neuropathy” at age 3 years.